This year the Digital Health Passport team started work to support children, young people and their carers with sickle cell disease. We teamed up with the Caribbean and African Health Network (CAHN) to run workshops and interviews with young patients, parents and carers to explore what they would want from a digital health app.

From the outset it was apparent that these sessions were going to be more emotive and distressing than other workshops on long-term conditions we had run. Hearing the personal experiences of people living with sickle cell disease was humbling, powerful and enlightening. The impact the disease has on people and on families looking after frightened children in extreme pain cannot easily be put into words and we’re extremely grateful to all the participants who took the time to share their stories. The knowledge that healthcare services fall short for these patients is not new, so it was motivating to understand how exactly we could make small but impactful changes.

The sessions were expertly facilitated by Dr Gertrude Wafula alongside Marta Denkiewicz (senior user researcher at Digital Health Passport) and attended by young people, parents and carers. We explored challenges, needs and ideas for where a digital service could help.

A summary of the key findings follows.

Struggles

The workshops identified several pain points experienced by sickle cell patients and their carers:

Difficulty in managing unpredictability: The unpredictable nature of sickle cell attacks makes it challenging to effectively manage the condition.

Impact on all aspects of life: Sickle cell affects all aspects of patients’ lives, including their relationships, social life, and career.

Mental health challenges: The ongoing management of sickle cell, the awareness of it being a lifelong condition, and the potential tragic consequences of a crisis have a significant impact on the mental health of patients and carers.

Lack of understanding and poor healthcare services: Sickle cell patients often face a lack of understanding regarding their condition, leading to difficulties in accessing essential services like care and education. This lack of awareness and stigma contributes to poor-quality healthcare services for these patients.

A digital service for sickle cell disease should provide…

Access to Reliable Information: many people struggled to find trusted information about the condition and treatments and had to do their own research. https://www.ciannassmile.co.uk/ was mentioned as an excellent resource alongside the https://www.sicklecellsociety.org/

What really helped was finding the charity Cianna’s Smile – every other information was focusing on the negative, your child will be sick all the time, your child will be in hospital a lot, and at the time that was not what I was looking for, but learning from other families and that community and the charity rather than GP and nurse was much more helpful.

Better Support During Initial Diagnosis: the emotional shock during the initial diagnosis makes it challenging for patients and carers to absorb the information provided at that time. There is a lack of consistency in the way information is conveyed or available to patients at home.

Peer support: participants found peer support to be the most useful source of information, allowing them to learn from individuals who have experienced similar situations. Peer support provided both medical and emotional advice. Dr Wafula also shared the details of the counselling service provided by CAHN https://www.cahn.org.uk/counselling-service/

Pain tracking: people would benefit from the ability to track and share their pain experiences with clinicians at appointment and during emergency department admissions.

Trigger recording: people should be able to learn about and track triggers to better manage their condition and get alerts for things such as weather changes.

Appointment reminders and access to blood test results: Participants want convenient access to other health services within the app

User profile: A section where app users can save basic information about themselves and their condition for emergency situations.

Well-being content and reminders: The inclusion of general well-being and mental health advice specific to sickle cell patients alongside nudges and reminders to do things

Additional languages: Content in multiple languages would help overcome language barriers for some patients.

Information sharing for carers: Tools facilitating information sharing between carers, such as spouses, were also desired.

What is sickle cell disease?

Sickle cell disorder is an inherited condition affecting haemoglobin in red blood cells, responsible for carrying oxygen and giving cells their red colour. It’s not contagious but is inherited when both parents pass on the sickle cell gene. The disorder causes anaemia and painful episodes known as sickle cell crises, occurring when deoxygenated cells change shape and block blood vessels. Treatment focuses on managing pain, typically with strong painkillers like morphine, and preventing complications.

Complications include stroke, acute chest syndrome, blindness, organ damage, and priapism. Over time, it can lead to organ damage, including to the liver, kidneys, lungs, heart, and spleen, and can be fatal. Sickle cell is the most common genetic condition in the UK, named for the crescent moon shape of affected red blood cells. This shape causes rigidity and blood vessel blockages, leading to pain and potential organ damage.

Symptoms can vary but often include strokes (especially in children aged 2-16), blindness, organ failure, pulmonary hypertension, acute chest syndrome, leg ulcers, gallstones, and headaches. There are over 1,000 types of abnormal haemoglobin, but the most common in the UK are haemoglobin S, C, D, and beta thalassemia. It’s estimated that 1 in 7 adults of African and Caribbean descent in the UK carry the sickle cell gene, with over 15,000 individuals having the disorder and 250,000 carrying the trait.

Factors triggering sickling of red blood cells include dehydration, infection, excessive exercise, organ failure, stress, anxiety, and sudden temperature changes. Babies are tested for sickle cell in the UK using a heel-prick blood test within their first week of life.

What is already known?

The report ‘No-One’s listening’ (2021) was triggered by the Coroner’s report into the death of Evan Nathan Smith in North Middlesex hospital. The enquiry demonstrated:

Sub-Standard Care and Variation: Sickle cell patients often receive sub-standard care, with significant variations depending on staff and location.

Lack of Joined-Up Care: There is a notable lack of effective joined-up care for sickle cell patients. Communication failures between different hospital departments impact care, and patient care plans are often ignored.

Low Awareness and Training: There is a low level of awareness about sickle cell among healthcare professionals, often necessitating patients to educate them during times of pain and distress. This lack of awareness is attributed to inadequate training for trainee nurses and medics. As a result, patients are frequently treated with disrespect, not believed, or not treated as a priority, with evidence suggesting that racism plays a role in these negative attitudes​​.

Inadequate Investment: Sickle cell care suffers from inadequate investment. Services are under-resourced and under-staffed, with a lack of investment in research and treatments over decades​​.

Non-Adherence to National Care Standards: There is a lack of adherence to national care standards in sickle cell care, causing frustration among patients and clinicians.

Fear and Avoidance of Hospitals: Many sickle cell patients, due to their experiences of sub-standard care, fear accessing secondary care or avoid hospitals altogether.

The Sickle cell digital discovery report: Designing better acute painful sickle cell care (2022) commissioned by the NHS Race and Health Observatory set out to understand the broad availability of digital products and services that currently exist.

Researchers were struck by the limited amount of technology, dedicated health sites, and web or digital tools that currently exist to support patients at the point of crisis and for managing the condition at home. These included online medical care plans, apps, wearables and dedicated forum pages.

Where apps for sickle cell disease were identified, these were highly variable in quality and subject to poor user interaction, not widely downloaded nor regularly updated.

What is the Digital Health Passport?

The Digital Health Passport (DHP) is an app to support self-management of long-term conditions that develop in childhood. It has been co-designed with young people, parents, carers, and professionals. Starting with asthma and allergies, the digital service is now being expanded to support families and young people with epilepsy, sickle cell disease and other long-term conditions.

Everything in One Place
Simplifies self-management by consolidating all necessary tools, information, and support into a single, easily accessible place.

Personalised Care Plans
A digital home for a personal action plan, making it easy to share emergency instructions with friends and family.

Medication Reminders
Supports adherence to medication regimes, thereby improving overall symptom control.

Symptom and Peak Flow Monitoring
Enables early detection of worsening symptoms, allowing for timely medical intervention.

Trigger Avoidance
Provides air quality, weather and pollen alerts, helping users avoid environmental triggers and manage their health more effectively.

Support
Connects users to peer support, charity helplines, and NHS urgent care services, ensuring they get the right care at the right time.

Educational Materials
Offers educational content from trusted sources like NHS.uk, Asthma & Lung UK, Young Epilepsy, and the Anaphylaxis Campaign enhancing users’ skills, knowledge, and confidence in self-managing their condition.

NHS login
In commissioned areas patients can use NHS login to access their GP record, order repeat medications and book appointments (coming soon!).

What does this work add?

Sickle Cell Disease presents a significant healthcare challenge that demands attention and tailored solutions. By developing a robust app that offers comprehensive support and reliable information, we aim to empower patients and carers, addressing key needs while also fostering a sense of community. Together with organisations like CAHN, we are committed to making a positive impact in the lives of sickle cell patients and working towards reducing the disparities and challenges they currently face.

These sessions are part of the co-design process to ensure that the development of the Digital Health Passport for Sickle Cell meets the needs of young people, patients and carers. The workshops provided the foundation to move forward iterating the design, curating the content and implementing the new features.

The team and their collaboration partners have now developed a prototype app with many of the features requested and are proceeding to complete the final version for regulatory approval and use in the NHS.

Alongside the design work, another workstream has progressed to demonstrate interoperability with the care plan system used in London (the Universal Care Plan).

The project team and their partners have now completed the feasibility, design and initial development of the digital service and are now in a position to seek support of senior NHS decision makers to fund the service through to national implementation.

Thanks to

All the participants who attended the workshops and interview sessions.
Caribbean & African Health Network, with special mention to Dr Gertrude Wafula.
NIHR i4i FAST grant for funding this work.

If you would like to work with us or learn more about the findings from our research, reach out to us on [email protected]